Using 42 strength and functional assessments recorded monthly, the natural history of amyotrophic lateral sclerosis (ALS) is described in 167 patients (98 men, 67 women) followed in five medical centers in the western United States. The mean age at onset was 57.4 years, and symptoms were present for 2.64 years before study entry. Although there was a highly variable rate of decline within the group of patients, there were no differences in rate of decline by age or gender. Older patients and women were weaker on entry. Forty-eight patients died during the study. The median survival was 4.0 years for the study cohort but 2.1 years for newly diagnosed cases. Decline in pulmonary function most closely correlated with death. Our results emphasize the importance of considering clinical variability in planning clinical trials. One possible strategy is to identify and stratify patients by rate of decline in pulmonary function since prospectively identifying homogeneous subgroups allows investigators to substantially reduce sample size in therapeutic trials.
This paper presents findings of a preliminary investigation of the relationship between upper limb muscle loading, reported discomfort, and clinical findings associated with keyboard use. Surface electromyography (SEMG) was recorded bilaterally from the digit flexors (FDS), digit extensors (EDC), and m. trapezius of 52 employees of a large insurance company during their normal work activities involving keyboarding.Normalized SEMG amplitude served as a measure of loading on the relevant muscle groups. Physical examinations were conducted on these employees within 14 days of the EMG recording session, as was the administration of a discomfort questionnaire.The EMG measures of upper limb muscle loading did not predict MSD cases as well as the presence of current symptoms for forearm extensor cases (OR = 9.66), the duration of symptoms for neck/shoulder cases (OR = 2.5), or the intensity of symptoms for forearm flexor cases (OR = 3.72). However, the prediction of forearm extensor case status was improved by including the median EDC normalized EMG (OR = 1.39). The prediction of forearm flexor and neck/shoulder cases was not appreciably improved by including the FDS and trapezius EMG loading variables.
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